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Biology subjects

Weng, C.

Publications and source records attributed to Weng, C..

At least 19 recordsLinked to original sources

Multimodal allostery in a single-domain protein

Allosteric communication between distant sites in proteins is central to biological regulation and underlies the efficacy of many drugs. Allostery allows proteins to function as molecular switches, with their activities controlled by ligand binding, covalent modifications, and mutations outside their active sites. Allosteric maps have been constructed for several proteins, but these all quantify regulation of a single site in each protein. How the allosteric maps of different sites compare is unclear. Here, we address this question by charting complete allosteric maps for two structurally distant binding sites in the oncoprotein KRAS. Unexpectedly, these maps reveal three different forms of allosteric control, that we term coupled, anti-coupled and independent allostery. Allostery in this small protein is therefore complex and multimodal. The co-existence of multiple allosteric networks in a single-domain protein has important conceptual implications and, if widespread, may allow the development of new classes of therapeutics that not only modulate targets but also tune their functional outputs.

biochemistry↗

A functional genomics screen of human B-cell differentiation reveals convergent mechanisms of inherited childhood leukemia predisposition

B-cell acute lymphoblastic leukemia (B-ALL) is the most common childhood cancer, yet the mechanisms by which inherited risk variants predispose to leukemia development remain poorly understood. A major challenge to studying these mechanisms has been the lack of model systems that faithfully capture the transient developmental states in which predisposition alleles are thought to act. Here, we establish a human B-cell differentiation platform from hematopoietic stem/progenitor cells that enables CRISPR-based engineering, recapitulates early B-cell lymphopoiesis, and enriches for rare developmental intermediates. By applying systematic perturbations with multiplexed single-cell transcriptomic profiling to mimic the effects of mutations in nine familial B-ALL predisposition genes, we decipher mechanisms by which B-cell development can be altered by such inherited variation to predispose to B-ALL. Through these studies, we identify convergent delays in B-cell differentiation at progenitor stages characterized by high-level RAG1/2 recombination activity. We propose that these delays at progenitor stages increase the likelihood that cells can undergo illegitimate RAG-mediated recombination to promote transformation, a finding consistent with similar rates of illegitimate RAG-associated genomic alterations in those with B-ALL associated with familial predisposition variants compared to sporadic cases.

cancer biology↗

Single-cell multiomic mapping of genetic predisposition to childhood B-cell acute lymphoblastic leukemia

Inherited genetic variation substantially increases the risk for developing childhood B-cell acute lymphoblastic leukemia (B-ALL), the most common cancer in children, yet the underlying mechanisms remain poorly understood. To address this limitation, we employ a single-cell multiomic framework to functionally dissect common regulatory variants associated with B-ALL risk. Coupling this multiomic analysis with assessment of allelic skews in chromatin accessibility, we reveal the impact of risk alleles and disruptions in transcription factor networks specific to B-cell progenitors, thereby providing mechanistic insights into altered regulatory programs underlying B-ALL predisposition. By constructing long-range variant-to-target gene maps, we identify 34 high-confidence B-ALL susceptibility genes. Among these, we uncover and functionally validate a risk allele that selectively upregulates expression of ELK3, a previously unrecognized regulator of B-cell development and leukemogenesis. Together, these findings establish a comprehensive variant-to-function map of cell state-specific regulatory disruptions underlying inherited predisposition to B-ALL and define new risk mechanisms, which could pave the way for future targeted prevention approaches.

cancer biology↗

Mapping genetic risk mechanisms for immune-mediated diseases across human dendritic cell differentiation

Defining the cell types and mechanisms through which genetic variation operates is essential to understand the biological basis of disease. Although human dendritic cells (DCs) are crucial in regulating immunity, their rarity and limitations in available genomic data have hampered efforts to link inherited disease risk to specific DC subsets. Here, we present a single-cell multi-omic atlas of human DC differentiation from hematopoietic stem and progenitor cells (HSPCs) that includes chromatin accessibility and transcriptomic profiles to infer regulatory networks across DC subsets. By integrating this regulatory architecture with fine-mapped variants from hundreds of complex-trait genome-wide association studies, we systematically map inflammatory, autoimmune, and oncologic disease risk to DC subset-specific variant-to-gene mechanisms. For example, we identify a risk-associated variant that enhances the activity of a PLD4 regulatory element in plasmacytoid DCs, thereby increasing the risk of developing systemic lupus erythematosus and other immune disorders. Collectively, these findings enable a deeper understanding of how complex immune diseases can emerge due to the impact of genetic variation acting in specific DC subsets.

genetics↗

Modeling mitochondrial inheritance enables high-precision single-cell lineage tracing in humans

Somatic mutations in mitochondrial DNA (mtDNA) provide natural barcodes that enable engineering-free lineage tracing in human tissues, but the complex dynamics of mtDNA inheritance across cell divisions and incomplete sampling of mtDNA introduce uncertainty in reconstructed lineages. Here, we present MitoDrift, a probabilistic framework that integrates Wright-Fisher drift dynamics with sparse single-cell measurements to produce confidence-refined lineage trees enriched for accurate clonal relationships. Validation with gold-standard lentiviral barcoding and whole-genome sequencing demonstrates that MitoDrift outperforms existing tree reconstruction methods in precision while maintaining high clonal recovery, enabling robust analyses linking lineage to cell state. Applying MitoDrift to human hematopoiesis reveals an age-associated decline in clonal diversity with differential impact across cell types and identifies heritable regulatory programs in hematopoietic stem cells in vivo, linking AP-1/stress-associated programs to clonal expansions. In multiple myeloma, MitoDrift captures therapy-associated clonal remodeling undetectable by copy number analysis, revealing phenotypic transitions and linking gene regulatory programs to differential drug sensitivity. Collectively, MitoDrift enables high-precision lineage tracing at scale and establishes quantitative lineage-state analysis in primary human tissues, linking clonal history to transcriptional and epigenetic programs in tissue homeostasis, aging, and disease.

genomics↗

Senescent host-derived extracellular vesicles potentiate antitumor immunity via bystander T cell activation

Extracellular vesicles (EVs) are key mediators of intercellular communication, and those from the plasma of aged individuals are typically viewed as drivers of aging or even cancer. Here, we uncover a latent, potent antitumor capacity inherent to EVs derived from aged hosts. Specifically, we demonstrate that extracellular vesicles from the plasma of aged mice (A-EVs) exhibit robust tumor-suppressive activity in young tumor-bearing recipients. Mechanistically, A-EVs engage a dual mode of action: they directly induce senescence in cancer cells, enhancing their immunogenicity. More importantly, they concurrently activate non-tumor-specific bystander CD8 T cells within the tumor microenvironment via cross-presentation of pathogen-derived antigens, thereby reshaping the immunosuppressive tumor microenvironment. This synergistic interplay potently reinvigorates antitumor immunity and sensitizes tumors to PD-L1 checkpoint blockade. Our findings establish A-EVs as a novel class of endogenous immunomodulators with broad therapeutic potential for cancer immunotherapy.

immunology↗

Genetic variation reveals a homeotic long noncoding RNA that modulates human hematopoietic stem cells

The HOXA gene locus coordinates body patterning, hematopoiesis, and differentiation. While studying blood phenotype-associated variation within the HOXA locus, we identified a genetic variant, rs17437411, associated with globally reduced blood counts, protection from blood cancers, and variation in anthropometric phenotypes. We find that this variant disrupts the activity of a previously unstudied antisense long non-coding RNA (lncRNA) located between HOXA7 and HOXA9, which we have named HOTSCRAMBL. The HOTSCRAMBL variant disrupts lncRNA function and reduces human hematopoietic stem cell (HSC) self-renewal. Mechanistically, HOTSCRAMBL enables appropriate expression and splicing of HOXA genes in HSCs, most notably HOXA9, in an SRSF2-dependent manner. Given the critical role of HOXA gene expression in some blood cancers, we also demonstrate that HOTSCRAMBL variation or deletion compromises HOXA-dependent acute myeloid leukemias. Collectively, we show how insights from human genetic variation can uncover critical regulatory processes required for effective developmental gene expression.

genetics↗

Leveraging mRNA platform for the development of vaccines against egg allergy

Food allergy has posed a major global health burden due to the rising prevalence and lack of effective prophylactic strategies. During the past decades, allergen-specific immunotherapy (AIT) has been used as a disease-modifying therapy for food allergic conditions, but due to the long-term treatment duration, poor patient compliance and unexpected adverse reactions, only a minority of patients benefit from AIT therapy. In this proof-of-concept study, using well-established mRNA platform, we developed mRNA vaccine candidates encoding for the major egg white allergen Gal d2 (also known as ovalbumin) and comprehensively evaluated the prophylactic efficacy against anaphylaxis in a Gal d2-induced allergic mouse model. Two vaccine formulations, Gal d2 mRNA vaccine and Gal d2-IL-10 mRNA vaccine, both demonstrated effective ability in preventing the onset of allergic disease, which is largely attributed to the versatilities of mRNA vaccine in eliciting allergen-blocking antibody, shifting Th2 towards Th1 immunity, as well as in generating peripheral tolerance.

immunology↗

Path of differentiation defines human macrophage identity

Macrophages play central roles in immunity, wound healing, and homeostasis - a functional diversity that is underpinned by varying developmental origins. The impact of ontogeny on properties of human macrophages is inadequately understood. We demonstrate that definitive human fetal liver (HFL) hematopoietic stem cells (HSCs) possess two divergent paths of macrophage specification that lead to distinct identities. The monocyte-dependent pathway exists in both prenatal and postnatal hematopoiesis and generates macrophages with adult-like responses properties. We now uncover a fetal-specific pathway of expedited differentiation that generates tissue resident-like macrophages (TRMs) that retain HSC-like self-renewal programs governed by the aryl hydrocarbon receptor (AHR). We show that AHR antagonism promotes TRM expansion and mitigates inflammation in models of atopic dermatitis (AD). Overall, we directly connect path of differentiation with functional properties of macrophages and identify an approach to promote selective expansion of TRMs with direct relevance to inflammation and diseases of macrophage dysfunction.

molecular biology↗

Robustness and reliability of single-cell regulatory multi-omics with deep mitochondrial mutation profiling

The detection of mitochondrial DNA (mtDNA) mutations in single cells holds considerable potential to define clonal relationships at scale, coupled with information on cell state in humans. Previous methods focused on higher heteroplasmy mutations, which, while informative, are few in number and may be shaped by functional selection, providing limited lineage information and potentially introducing biases for tracing. Although more challenging to detect, intermediate- to low-heteroplasmy mtDNA mutations are valuable due to their high diversity, abundance, and lower propensity to selection. To enhance mtDNA mutation detection and facilitate fine-scale lineage tracing, we developed the single-cell Regulatory multi-omics with Deep Mitochondrial mutation profiling (ReDeeM) approach, an integrated experimental and computational framework. Here, we specifically address two analytical challenges central to single-cell mtDNA-based lineage analysis: the fidelity of variant-calling workflows and the reliability of phylogenetic inference. We demonstrate that, by leveraging consensus-based error correction, ReDeeMs mtDNA mutation calls achieve high fidelity, aligning with bona fide mutational signatures even for mutations supported by a single molecule per cell. We also developed an improved post-consensus filtering approach, termed "filter2" that systematically identifies and filters residual edge-enriched artifacts, even though these affect only a minority of mutation calls. To systematically validate ReDeeM, we recently conducted a lentiviral barcoding experiment in human HSCs, uniquely labeling each cell prior to expansion and differentiation to provide a ground truth for assessing lineage tracing accuracy1. Such validation demonstrate that the original ReDeeM analytic approach (filter1)2 robustly recovers true clonal structure at high resolution and recall. Including intermediate to low-heteroplasmy variants (<10% per cell) strongly improves lineage inference, whereas excluding mutations supported by a single molecule per cell removes true clonal signal and degrades recovery of true clones. Both filter1 and filter2 accurately recover true clones and outperform prior mtDNA-based lineage tracing approaches in ground-truth precision and recall, with filter2 providing additional gains in performance. Finally, while ReDeeM advances mtDNA-based lineage tracing by recovering true clones at high resolution, yet quantifying the phylogenetic uncertainty from mitochondrial inheritance remains unmet. To address this, we recently developed and validated MitoDrift1 as a next-generation, drift-aware framework for mtDNA lineage tracing that further strengthens phylogenetic inference and provides interpretable uncertainty estimates.

genomics↗

The functional and pathogenic consequences of fibrinogen on human oligodendroglia

Fibrinogen is a blood-derived protein involved in coagulation, and can make its way into the central nervous system (CNS) following breakdown of the blood-brain barrier. This molecule has been implicated in multiple sclerosis (MS), a disease marked by inflammation and demyelination in the CNS, as well as other neurological disorders. However, the effect of this molecule has not been studied on human myelinating cells. This study examines how fibrinogen influences human oligodendrocyte (OL) lineage cells at various stages of development. Using induced pluripotent stem cell-derived (iPSC) OL precursors and human primary OLs, we examined the effects of fibrinogen on cell differentiation, viability and myelination-related function. Here we show that fibrinogen induces an aberrant differentiation of early lineage OLs, by inhibiting their maturation and inducing an astrocytic phenotype, as seen in previous studies. On mature OLs, fibrinogen was found to promote myelination capacity as shown by ensheathment assays as well as on the RNA level. These effects were associated with the activation of BMP signalling, both in early and mature OLs. Transcriptomic analysis of human MS brain tissue shows similar pro-myelination changes in a subset of OLs, suggesting in vivo relevance. These findings indicate that fibrinogen has a lineage-dependent effect, where it may be inhibitory earlier in the lineage while promoting OL function in later stages. Understanding this dual role will provide insight into remyelination failure in MS and highlights the importance of timing and target in future therapeutic strategies. Significance StatementIn multiple sclerosis (MS), the blood protein fibrinogen leaks into the brain and has been shown to interfere with myelin repair. This study demonstrates that fibrinogen has opposite effects on human oligodendrocyte-lineage cells depending on their stage of maturation. While it blocks the differentiation of early-stage cells, it enhances the functional capacity of mature oligodendrocytes. These findings help explain why remyelination may fail in MS and suggest that fibrinogen could both hinder and support repair, depending on the cell context. This dual role has important implications for developing stage-specific therapies for MS.

neuroscience↗

Fine-tuning Large Language Models for Rare Disease Concept Normalization

ObjectiveWe aim to develop a novel method for rare disease concept normalization by fine-tuning Llama 2, an open-source large language model (LLM), using a domain-specific corpus sourced from the Human Phenotype Ontology (HPO). MethodsWe developed an in-house template-based script to generate two corpora for fine-tuning. The first (NAME) contains standardized HPO names, sourced from the HPO vocabularies, along with their corresponding identifiers. The second (NAME+SYN) includes HPO names and half of the concepts synonyms as well as identifiers. Subsequently, we fine-tuned Llama2 (Llama2-7B) for each sentence set and conducted an evaluation using a range of sentence prompts and various phenotype terms. ResultsWhen the phenotype terms for normalization were included in the fine-tuning corpora, both models demonstrated nearly perfect performance, averaging over 99% accuracy. In comparison, ChatGPT-3.5 has only [~]20% accuracy in identifying HPO IDs for phenotype terms. When single-character typos were introduced in the phenotype terms, the accuracy of NAME and NAME+SYN is 10.2% and 36.1%, respectively, but increases to 61.8% (NAME+SYN) with additional typo-specific fine-tuning. For terms sourced from HPO vocabularies as unseen synonyms, the NAME model achieved 11.2% accuracy, while the NAME+SYN model achieved 92.7% accuracy. ConclusionOur fine-tuned models demonstrate ability to normalize phenotype terms unseen in the fine-tuning corpus, including misspellings, synonyms, terms from other ontologies, and laymens terms. Our approach provides a solution for the use of LLM to identify named medical entities from the clinical narratives, while successfully normalizing them to standard concepts in a controlled vocabulary.

bioinformatics↗

Herpes zoster mRNA vaccine induces superior vaccine immunity over licensed vaccine in mice and rhesus macaques

Herpes zoster remains an important global health issue and mainly occurs in aged and immunocompromised individuals with an early exposure history to Varicella Zoster Virus (VZV). Although the licensed vaccine Shingrix has a remarkably high efficacy, undesired reactogenicity and increasing global demand causing vaccine shortage urged the development of improved or novel VZV vaccines. In this study, we developed a novel VZV mRNA vaccine candidate (named as ZOSAL) containing sequence-optimized mRNAs encoding full-length glycoprotein E encapsulated in an ionizable lipid nanoparticle. In mice and rhesus macaques, ZOSAL demonstrated superior immunogenicity and safety in multiple aspects over Shingrix, especially in the induction of strong T cell immunity. Transcriptomic analysis revealed that both ZOSAL and Shingrix could robustly activate innate immune compartments, especially Type-I IFN signaling and antigen processing/presentation. Multivariate correlation analysis further identified several early factors of innate compartments that can predict the magnitude of T cell responses, which further increased our understanding of the mode of action of two different VZV vaccine modalities. Collectively, our data demonstrated the superiority of VZV mRNA vaccine over licensed subunit vaccine. The mRNA platform therefore holds prospects for further investigations in next-generation VZV vaccine development.

immunology↗

Spateo: multidimensional spatiotemporal modeling of single-cell spatial transcriptomics

Cells do not live in a vacuum, but in a milieu defined by cell-cell communication that can be measured via emerging high-resolution spatial transcriptomics approaches. However, analytical tools that fully leverage such data for kinetic modeling remain lacking. Here we present Spateo (aristoteleo/spateo-release), a general framework for quantitative spatiotemporal modeling of single-cell resolution spatial transcriptomics. Spateo delivers novel methods for digitizing spatial layers/columns to identify spatially-polar genes, and develops a comprehensive framework of cell-cell interaction to reveal spatial effects of niche factors and cell type-specific ligand-receptor interactions. Furthermore, Spateo reconstructs 3D models of whole embryos, and performs 3D morphometric analyses. Lastly, Spateo introduces the concept of "morphometric vector field" of cell migrations, and integrates spatial differential geometry to unveil regulatory programs underlying various organogenesis patterns of Drosophila. Thus, Spateo enables the study of the ecology of organs at a molecular level in 3D space, beyond isolated single cells.

systems biology↗

The energetic and allosteric landscape for KRAS inhibition

Thousands of proteins have now been genetically-validated as therapeutic targets in hundreds of human diseases. However, very few have actually been successfully targeted and many are considered undruggable. This is particularly true for proteins that function via protein-protein interactions: direct inhibition of binding interfaces is difficult, requiring the identification of allosteric sites. However, most proteins have no known allosteric sites and a comprehensive allosteric map does not exist for any protein. Here we address this shortcoming by charting multiple global atlases of inhibitory allosteric communication in KRAS, a protein mutated in 1 in 10 human cancers. We quantified the impact of >26,000 mutations on the folding of KRAS and its binding to six interaction partners. Genetic interactions in double mutants allowed us to perform biophysical measurements at scale, inferring >22,000 causal free energy changes, a similar number of measurements as the total made for proteins to date. These energy landscapes quantify how mutations tune the binding specificity of a signalling protein and map the inhibitory allosteric sites for an important therapeutic target. Allosteric propagation is particularly effective across the central beta sheet of KRAS and multiple surface pockets are genetically-validated as allosterically active, including a distal pocket in the C-terminal lobe of the protein. Allosteric mutations typically inhibit binding to all tested effectors but they can also change the binding specificity, revealing the regulatory, evolutionary and therapeutic potential to tune pathway activation. Using the approach described here it should be possible to rapidly and comprehensively identify allosteric target sites in many important proteins.

biophysics↗

Spatial compartmentalization of signalling imparts source-specific functions on secreted factors

Efficient regeneration requires multiple cell types acting in a coordination. To better understand the intercellular networks involved and how they change when regeneration fails, we profiled the transcriptome of hematopoietic, stromal, myogenic, and endothelial cells over 14 days following acute muscle damage. A time-resolved computational model of interactions was generated, and VEGFA-driven endothelial engagement was identified as a key differentiating feature in models of successful and failed regeneration. In addition, it revealed that the majority of secreted signals, including VEGFA, are simultaneously produced by multiple cell types. To test whether the cellular source of a factor determines its function, we deleted VEGFA from two cell types residing in close proximity, stromal and myogenic progenitors. By comparing responses to different types of damage, we found that myogenic and stromal VEGFA have distinct functions in regeneration. This suggests that spatial compartmentalization of signaling plays a key role in intercellular communication networks. HighlightsO_LILigand-receptor signaling redundancy during skeletal muscle regeneration C_LIO_LIInflammatory cells, and muscle and fibro/adipogenic progenitors produce VEGFA C_LIO_LIVEGFA from muscle progenitors control their proliferation after muscle damage C_LIO_LIVEGFA from FAP controls angiogenesis only after ischemic damage C_LI eTOC blurbGroppa et al. performed a novel time-resolved bioinformatics analysis that revealed extensive ligand-receptor redundancy among the cell types contributing to skeletal muscle regeneration. They focused on one of these pathways, and showed that VEGFA from different cell types has distinct roles in regeneration.

cell biology↗

Variant to function mapping at single-cell resolution through network propagation

With burgeoning human disease genetic associations and single-cell genomic atlases covering a range of tissues, there are unprecedented opportunities to systematically gain insights into the mechanisms of disease-causal variation. However, sparsity and noise, particularly in the context of single-cell epigenomic data, hamper the identification of disease- or trait-relevant cell types, states, and trajectories. To overcome these challenges, we have developed the SCAVENGE method, which maps causal variants to their relevant cellular context at single-cell resolution by employing the strategy of network propagation. We demonstrate how SCAVENGE can help identify key biological mechanisms underlying human genetic variation including enrichment of blood traits at distinct stages of human hematopoiesis, defining monocyte subsets that increase the risk for severe coronavirus disease 2019 (COVID-19), and identifying intermediate lymphocyte developmental states that are critical for predisposition to acute leukemia. Our approach not only provides a framework for enabling variant-to-function insights at single-cell resolution, but also suggests a more general strategy for maximizing the inferences that can be made using single-cell genomic data.

genomics↗

Neptune: An environment for the delivery of genomic medicine

PurposeGenomic medicine holds great promise for improving healthcare, but integrating searchable and actionable genetic data into electronic health records remains a challenge. Here, we describe Neptune, a system for managing the interaction between a clinical laboratory and an electronic health record system. MethodsWe developed Neptune and applied it to two clinical sequencing projects that required report customization, variant reanalysis and EHR integration. ResultsNeptune enabled the analysis of data for generation of and delivery to EHR systems of over 15,000 clinical genomic reports. These projects demanded customizable clinical reports that contained a variety of genetic data types including SNVs, CNVs, pharmacogenomics and polygenic risk scores. Two variant reanalysis activities were also supported, highlighting this important workflow. ConclusionsMethods are needed for delivering structured genetic data to EHRs. This need extends beyond developing data formats to providing infrastructure that manages the reporting process itself. Neptune was successfully applied on two high-throughput clinical sequencing projects to build and deliver clinical reports to EHR systems. The software is open and available at https://gitlab.com/bcm-hgsc/neptune.

bioinformatics↗